Monoclonal Gammopathy

• Plasma cell disorders
  • Monoclonal gammopathy of undetermined significance (MGUS)
  • Biclonal gammopathy of undetermined significance
  • Idiopathic Bence Jones proteinuria
  • POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome
  • Osteosclerotic myeloma
  • Castleman's disease
  • AL (light chain) amyloidosis, light chain and heavy chain deposition diseases
  • Solitary plasmacytoma
  • Multiple myeloma, smoldering multiple myeloma
    

    

• B-cell lymphoproliferative disorders
  • Non-Hodgkin's lymphoma
  • Chronic lymphocytic leukemia
  • Lymphoplasmacytic lymphoma (Waldenstrom macroglobulinemia)
  • Post-transplant monoclonal gammopathies
  • Heavy chain diseases
• Connective tissue disorders
  • SLE
  • RA
  • Sjogren syndrome
  • Scleroderma
  • Psoriatic arthritis
  • Polymyalgia rheumatica
• Associated with infections
  • Hepatitis C
  • HIV/AIDS
• Dermatologic disorders
  • Sclerodema (scleromyxedema), Lichen myxoedematosus
  • Diffuse plane xanthomatosis
  • Urticaria and IgM (Schnitzler's syndrome)
  • Subcorneal pustular dermatosis
  • Necrobiotic xanthogranuloma
  • Pyoderma gangrenosum
• Miscellaneous disorders
  • Acquired von Willebrand disease
  • Acquired angioedema (acquired C1-INH deficiency)
  • Eosinophilic fasciitis
  • Cryoglobulinemia, cryofibrinogenemia
  • Myelodysplastic syndrome
  • Chronic neutrophilic leukemia
  • Sensorimotor neuropathy with MGUS
  • Capillary leak syndrome
  • T-cell large granular lymphocyte leukemia
  • Cold agglutinin disease

Polyclonal Gammopathy

In 148 patients with polyclonal gamma globulin level ≥3 g/dL:

• Liver disease (61%)
  • Most with autoimmune hepatitis
  • Rest included hepatitis C, hepatitis B, primary biliary cirrhosis, primary sclerosing cholangitis, ethanol-induced liver injury, cryptogenic cirrhosis, primary hemachromatosis, and alpha-1 antitrypsin deficiency
• Connective tissue disease (22%)
  • Sjögren syndrome most common, followed by RA, SLE, mixed CTD
• Chronic infection (6%)
  • Hepatitis C and B
  • HIV
    • Polyclonal gammopathy with bone marrow plasmacytosis is a common finding in HIV; these patients also have an increased incidence of clonal plasma cell disorders (eg, MGUS, multiple myeloma, plasmablastic lymphoma)
• Hematologic disorders (5%) - benign and malignant B and T lymphoproliferative disorders (including benign lymphoproliferative disorders, lymphomas, lymphocytic leukemias, myelodysplastic syndrome)
• Non-hematologic malignancy (3%) - hepatocellular carcinoma, ovarian carcinoma, squamous cell carcinoma of the lung, pancreatic adenocarcinoma, renal cell carcinoma, and chondrosarcoma

Elevated IgE

Immunoglobulin Reference Values

References